Introduction
Nevus lipomatosis cutaneous superficialis (NLCS) is a rare benign idiopathic dermal anomaly and was first reported by Hoffmann and Zurhelle in 1921 as pedunculated lipofibroma.1 Commonly they present at birth or within the first 3 decades of life but solitary form have a later onset in life. There is no hereditary predisposition or sex predilection of this disease.2 Clinically it occurs in two forms namely 1) classical and 2) solitary. Classical form is most frequent form with common presentation over pelvic-girdle area and manifests as clusters of multiple, pedunculated, yellowish or skin-colored, soft, non - tender, cerebriform, papules or nodules which may coalesce to form large plaques.2, 3 The second form is the solitary form which is rare and normally presents as a solitary dome-shaped papule or nodule with no specific site distribution.4, 5 Histologically, it is characterized by ectopic mature adipocytes within the upper dermis with no connection to underlying subcutaneous fat.2, 6
We report here 4 cases of NLCS to raise recognition of this rare disease.
Case 1
A 41 year old male presented to surgery outpatient department with a single swelling at left arm and provisional diagnosis of sebaceous cyst was made. An excisional biopsy was done. Grossly two firm to hard tissue pieces largest measuring 2.2x1.6x0.6 cm were received in histopathology department. Microscopic examination shows keratinized stratified squamous epithelium revealing hyperkeratosis, acanthosis and occasional horn cysts. Papillary and mid dermis shows the presence of mature adipose tissue embedded in the collagen bundles. Reticular dermis was unremarkable. On account of above histopathological features the diagnosis of Nevus lipomatosis superficialis was given.
Case 2
A 50 years female presented to surgery outpatient department with a single swelling at right thigh and provisional diagnosis of lipoma was made. An excisional biopsy was done. Grossly a single skin covered globular mass with fatty tissue measuring 1.0x1.5x0.6 cm was received in histopathology department. On cut open yellowish white areas were seen. Microscopic examination shows a tumor lined by keratinized stratified squamous epithelium with basket weave appearance of stratum corneum, thinning of epidermis and loss of rete ridges. Papillary dermis shows moderate to marked lymphocytic infiltrate. Papillary dermis was replaced by mature adipose tissue. Hair follicles, eccrine ducts and glands, smooth muscles and collagen bundles also noticed within the dermis. In addition, focal areas show fat necrosis. On account of above histopathological features the diagnosis of Nevus lipomatosis superficialis was given.
Case 3
A 29 years old girl presented to dermatology department with a soft pedunculated growth of size 3.0x1.0 cm at anterior surface of left thigh since 4 to 5 years. No family history of similar complaint was present. An excisional biopsy was done. Grossly a single skin covered firm tissue piece measuring 2.0x1.6x0.6cm was received in histopathology department with differentials of skin tag, nevus lipomatosis and warts. Microscopic examination shows keratinized stratified squamous epithelium revealing basket weave appearance of stratum corneum, follicular plugging, mild acanthosis and increased melanin pigment in the basal layer. Throughout dermis shows lobules of mature adipocytes separated by collagen fibres On account of above histopathological features the diagnosis of Nevus lipomatosis superficialis was given after exclusion of other differentials.
Case 4
A 30 years female presented to surgery outpatient department with a single swelling with stalk over back and provisional diagnosis of papilloma was made. An excisional biopsy was done. Grossly a single skin covered globular multilobulated mass with fatty tissue measuring 1.8x1.5x0.8 cm was received in histopathology department. On cut open yellowish white areas were seen. Microscopic examination shows a polypoidal lesion covered by atrophic stratified squamous epithelium with basket weave appearance of stratum corneum, Papillary and reticular dermis shows variably sized lobules of mature adipose tissue. On account of above histopathological features the diagnosis of Nevus lipomatosis superficialis was given.
Discussion
Nevus lipomatosus superficialis is an idiopathic benign cutaneous anomally with a rare occurence having two clinical forms namely 1) classical Hoffmann-Zurhelle form and 2) solitary form. 1 Triki et al (Tunisia, 2006) noticed an increased prevalence of the solitary form3 which frequently develops as a single nodule after 20 years of age having no sites of predilection and has been demonstrated on the arms, face, nose, knees, calf, ears, clitoris, axillae and scalp. 2, 4, 5, 6, 7, 8 The classical form/Hoffmann-Zurhelle form occuring at birth or first 3 decades of life 2, 7, 9 presents as unilateral clusters of multiple, soft, pedunculated-cerebriform, yellow/skin colored papular or nodular lesion which usually coalesce forming plaque. They are most commonly seen on the pelvic girdle area with a zonal pattern. 2, 3
All the patients in these case series of four cases typically had a solitary form with single nodular lesion on the left arm, right thigh and left thigh respectively. The differential diagnosis for this condition includes skin tag (papillomas), connective tissue nevus, plexiform neurofibroma, vascular malformation, nevus sebaceous and lipoblastomatosis.
All these 4 cases had a provisional diagnosis of sebaceous cyst, lipoma, skin tag/warts and papilloma respectively and final diagnosis of nevus lipomatosis superficialis was made by histopathological examination which helped in distinguishing this condition from rest of the differentials.
Conclusion
As nevus lipomatosus superficialis is an idiopathic rare benign cutaneous condition requiring an expertise - first, to suspect this benign condition and secondly histopathological examination is must to reach a diagnosis. Excision is the ultimate cure for this condition with minimal chances of recurrence.
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